title: Delay in recognition of pulmonary arterial hypertension: factors identified from the REVEAL Registry.
abstract: BACKGROUND
  Pulmonary arterial hypertension (PAH) is a progressive and fatal disorder. Despite the emergence of effective therapy, PAH is commonly at an advanced stage when recognized. Factors associated with a prolonged symptomatic period before the recognition of PAH have not been fully evaluated.
  
  
  METHODS
  The Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL Registry) enrolled 2,967 US adult patients with PAH from March 2006 to September 2007. Patients were considered to have delayed disease recognition if > 2 years elapsed between symptom onset and the patient receiving a PAH diagnosis, starting on PAH-specific therapy, or receiving a diagnosis by right-sided heart catheterization.
  
  
  RESULTS
  In 21.1% of patients, symptoms were experienced for > 2 years before PAH was recognized. Patients with onset of PAH symptoms before age 36 years showed the highest likelihood of delayed disease recognition (OR, 3.07; 95% CI, 2.03-4.66). History of obstructive airways disease (OR, 1.93; 95% CI, 1.5-2.47) and sleep apnea (OR, 1.72; 95% CI, 1.33-2.22) were independently associated with delayed PAH recognition. Six-minute walk distance < 250 m (OR, 1.91; 95% CI, 1.16-3.13), right atrial pressure < 10 mm Hg (OR, 1.77; 95% CI, 1.26-2.48), and pulmonary vascular resistance < 10 Wood units (OR, 1.28; 95% CI, 1.02-1.60) were also associated with delayed disease recognition, but sex, race/ethnicity, and geographic region showed no association.
  
  
  CONCLUSIONS
  One in five patients in the REVEAL Registry who were diagnosed with PAH reported symptoms for > 2 years before their disease was recognized. Younger individuals and patients with histories of common respiratory disorders were most likely to experience delayed PAH recognition.
  
  
  BACKGROUND
  ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov.
authors.affiliation: Department of Medicine, Division of Pulmonary and Critical Care Medicine, Intermountain Medical Center, Murray, UT; Department of Internal Medicine, Pulmonary Division, University of Utah, Salt Lake City, UT.
authors.forename: Lynette M
authors.initials: LM
authors.lastname: Brown
authors.affiliation: Department of Medicine, Pulmonary and Critical Care Division, University of California, San Francisco, CA.
authors.forename: Hubert
authors.initials: H
authors.lastname: Chen
authors.affiliation: Department of Medicine, Pulmonary, Allergy and Critical Care Division, University of Pennsylvania, Philadelphia, PA.
authors.forename: Scott
authors.initials: S
authors.lastname: Halpern
authors.affiliation: Department of Medicine, Pulmonary, Allergy and Critical Care Division, University of Pennsylvania, Philadelphia, PA.
authors.forename: Darren
authors.initials: D
authors.lastname: Taichman
authors.affiliation: Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN.
authors.forename: Michael D
authors.initials: MD
authors.lastname: McGoon
authors.affiliation: Pulmonary, Allergy, Sleep, and Critical Care Medicine, Boston University Medical Center, Boston, MA.
authors.forename: Harrison W
authors.initials: HW
authors.lastname: Farber
authors.affiliation: Section of Pulmonary, Critical Care, and Sleep Medicine, Baylor College of Medicine, Houston, TX.
authors.forename: Adaani E
authors.initials: AE
authors.lastname: Frost
authors.affiliation: Department of Internal Medicine, Pulmonary Division, University of Utah, Salt Lake City, UT.
authors.forename: Theodore G
authors.initials: TG
authors.lastname: Liou
authors.affiliation: Statistical Analysis, ICON Clinical Research, San Francisco, CA.
authors.forename: Michelle
authors.initials: M
authors.lastname: Turner
authors.affiliation: Clinical Operations, Actelion Pharmaceuticals US, Inc, South San Francisco, CA.
authors.forename: Kathy
authors.initials: K
authors.lastname: Feldkircher
authors.affiliation: Statistical Analysis, ICON Clinical Research, San Francisco, CA.
authors.forename: Dave P
authors.initials: DP
authors.lastname: Miller
authors.affiliation: Department of Medicine, Division of Pulmonary and Critical Care Medicine, Intermountain Medical Center, Murray, UT; Department of Internal Medicine, Pulmonary Division, University of Utah, Salt Lake City, UT. Electronic address: greg.elliott@imail.org.
authors.forename: C Gregory
authors.initials: CG
authors.lastname: Elliott
mesh_terms: D000328:Adult; D000368:Aged; D000402:Airway Obstruction / Q000150:complications* / Q000175:diagnosis; D006328:Cardiac Catheterization / Q000379:methods*; D003937:Diagnosis, Differential; D018450:Disease Progression; D042241:Early Diagnosis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011379:Prognosis; D011669:Pulmonary Wedge Pressure; D012042:Registries; D012189:Retrospective Studies; D012891:Sleep Apnea Syndromes / Q000150:complications* / Q000175:diagnosis; D013997:Time Factors; D014481:United States / Q000453:epidemiology